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Rapidly Progressive Glomerulonephritis Associated With Anti-glomerular Basement Membrane Disease: A Rare Diagnosis.
Cureus
December 2024
Medicine, Faculdade de Medicina da Universidade do Porto (FMUP), Porto, PRT.
Anti-glomerular basement membrane disease is a rare small vessel vasculitis caused by the deposition of immunoglobulin G (IgG) autoantibodies in the basement membrane of glomerular capillaries and lung alveoli, leading to rapidly progressive renal failure and/or alveolar hemorrhage. We report the case of an 83-year-old female patient presenting with uremic symptoms, rapidly progressive kidney failure, and a high titer of anti-glomerular basement membrane antibodies. Given the urgent need for kidney replacement therapy, the substantial fibrosis and glomerular scarring observed in the kidney biopsy suggesting a chronic process, and the absence of pulmonary involvement, neither immunosuppressive treatment nor plasmapheresis was initiated, since a low likelihood of a favorable response to these interventions was expected.
View Article and Find Full Text PDFIs there a link between Hepatitis A virus and Guillain-Barré syndrome? A systematic review of case reports.
eNeurologicalSci
March 2025
Neurosciences Research Center, Poursina Hospital, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran.
Introduction: Guillain-Barré syndrome (GBS) is an inflammatory disorder of the peripheral nervous system, causing acute flaccid paralysis. There have been occasional reports linking Hepatitis A virus (HAV) to GBS. Here we aimed to evaluate the current literature on the association between GBS and HAV, exploring potential mechanisms and clinical implications.
View Article and Find Full Text PDFPan-neurofascin autoimmune nodoparanodopathy: A case report and literature review.
Medicine (Baltimore)
January 2025
Department of Neurology (Nerve-Muscle Unit), Reference Center for Neuromuscular Diseases "AOC," ALS Reference Center, University Hospitals of Bordeaux (Pellegrin Hospital), University of Bordeaux, Bordeaux, France.
Rationale: Locked-in syndrome (and its variant, completely locked-in state) generally has a high mortality rate in the acute setting; however, when induced by conditions such as acute inflammatory polyradiculoneuropathy, it may well be curable such that an attempt at cure should be systematically sought by clinicians.
Patient Concerns: A 52-year-old man presented with acute tetraparesia and areflexia, initially diagnosed as Guillain-Barré syndrome. Despite appropriate treatment, his condition deteriorated, evolving into a completely locked-in state.
Microangiopathic Hemolytic Anemia: A Rare Complication of Acute Pancreatitis.
Korean J Gastroenterol
January 2025
Section of Haematology, Department of Pathology and Laboratory Medicine, Aga Khan University, Karachi, Pakistan.
Microangiopathic hemolytic anemia (MAHA) is a rare subtype of hemolytic anemia characterized by elevated hemolytic markers and red blood cell destruction. Though uncommon, MAHA can occur as a complication of acute pancreatitis because of the associated inflammatory response. Patients with MAHA secondary to pancreatitis show favorable outcomes when treated with plasma exchange.
View Article and Find Full Text PDFHematopoietic stem cell transplantation as rescue therapy for refractory autoimmune retinopathy: a case report.
Front Immunol
January 2025
Department of Ophthalmology, National University Hospital, National University Health System, Singapore, Singapore.
Autoimmune retinopathy (AIR) is a rare, potentially blinding retinal disease that remains a challenging condition to manage when resistant to conventional immune-modulatory approaches. We report clinical and electrophysiological improvement in a 49-year-old patient who underwent an autologous hematopoietic stem cell transplant (aHSCT) for thymoma-associated AIR after experiencing progressive disease despite receiving periocular and systemic steroids, mycophenolate mofetil, baricitinib, tacrolimus, bortezomib, rituximab, plasmapheresis, and intravenous immunoglobulin. The aHSCT had two stages: (i) peripheral blood stem cell harvest following mobilization with cyclophosphamide and granulocyte colony-stimulating factor, and (ii) conditioning regimen with plasmapheresis, rituximab, cyclophosphamide, and anti-thymocyte globulin high-dose therapy, followed by autologous hematopoietic cell infusion of 5.
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